Burkitt

Burkitt refers to Denis Parsons Burkitt (1911–1993), an Irish surgeon who, while working in equatorial Africa, described a fast-growing jaw tumor in children in 1958. The tumor was later recognized as a form of non-Hodgkin lymphoma and named Burkitt lymphoma in his honor. The disease is an aggressive B-cell non-Hodgkin lymphoma that proliferates rapidly and requires prompt, intensive chemotherapy. It has three clinical variants: endemic (African), sporadic (non-endemic), and immunodeficiency-associated. The endemic form is common in equatorial Africa and often presents with facial bone involvement; the sporadic form occurs worldwide, typically with abdominal or pelvic masses; immunodeficiency-associated disease is seen in people with HIV/AIDS or other immune suppression.

Genetically, Burkitt lymphoma typically involves translocations of the MYC gene on chromosome 8 with immunoglobulin gene loci on 14, 2, or 22, most often t(8;14). This leads to constitutive MYC expression and very high cell turnover, with a starry-sky histology and near-100% Ki-67. Diagnosis rests on tissue biopsy with immunophenotyping (CD20+, CD10+, BCL6+, usually-negative BCL2) and molecular testing for MYC rearrangements; CNS and bone marrow involvement are important to assess.

Treatment is intensive chemotherapy, often with regimens such as CODOX-M/IVAC or equivalent, sometimes including rituximab and central nervous system prophylaxis. Outcomes are favorable in many settings if treated promptly, with cure rates high in endemic and sporadic pediatric cases and substantial, though variable, rates in adults and immunocompromised patients.